Aggressive Inflammatory Myofibroblastic Tumor of Distal Pancreas: A Diagnostic and Surgical Challenge.

An inflammatory myofibroblastic tumor (IMT) is a rare soft tissue neoplasm of unknown etiology. It is a slow-growing tumor of borderline malignant potential. Distant metastases and recurrence after complete excision are rare. Establishing a preoperative diagnosis is difficult because of its nonspecific clinic-radiological features. Although the majority of cases have been reported in the lungs, it can affect any part of the body. The pancreatic inflammatory myofibroblastic tumor is very rare and only 26 cases have been reported in the medical literature. These tumors mostly arise from the head of the pancreas, whereas occurrence in the body or tail region is rather unusual. Here, we report a case of a 55-year-old male patient with a locally advanced inflammatory myofibroblastic tumor arising from the pancreatic tail. Complete excision of tumor required multi-visceral resection (distal pancreaticosplenectomy with jejunal and colonic segmental resection). The diagnosis of inflammatory myofibroblast tumor was made on the basis of histopathology and immunohistochemistry.

Isolated renal hydatid disease: laparoscopic approach to an uncommon entity (case report).

Hydatidosis is a parasitic disease, endemic in various parts of the World. It frequently involves liver and lungs and, rarely, other organs as well. Isolated renal hydatidosis is a rare entity that accounts for less than 3% of all hydatid cases. Surgery remains the mainstay of treatment. We hereby report a case of isolated renal hydatid cyst involving left kidney that was managed by laparoscopic approach.

Tactics, techniques, and challenges in the management of giant choledochal cyst in adolescents and adults: a retrospective cohort study.

BACKGROUND: Choledochal cyst is a rare congenital anomaly manifesting as cystic dilatation of the biliary tree. This study presents our 5-year experience with giant choledochal cyst in adolescents and adults, focusing primarily on its clinical presentation, operative challenges, and pragmatic solutions. METHODS: A retrospective observational study was conducted on 58 adolescent and adult choledochal cyst patients who were managed at a tertiary care hospital. Giant choledochal cyst is defined as cyst with a maximum diameter of ≥ 10 cm. Demographic profile, clinical presentation, and surgical outcome of these patients were analyzed. RESULTS: A total of 12 patients with giant choledochal cyst were managed in our department in a duration of 5 years. The median age of patients with giant choledochal cyst was 20 years (range, 13-30 years) and male to female ratio was 1:2. Giant choledochal cysts were more symptomatic and 8 out of 12 presented with classic triad of abdominal pain, mass, and jaundice. One patient with giant choledochal cyst had metastatic cholangiocarcinoma. Eleven patients underwent surgical cyst excision. Surgery of the giant cyst was challenging and required technical modifications for safe excision. CONCLUSION: Giant choledochal cyst is an unusual entity that is rarely encountered beyond first decade of life. These cysts pose unique surgical challenges and require modification of the standard operative technique.

Nasal lobular capillary hemangioma.

Nasal lobular capillary hemangioma is a rare benign tumor of the paranasal sinuses. This lesion is believed to grow rapidly in size over time. The exact etiopathogenesis is still a dilemma. We discuss a case of nasal lobular capillary hemangioma presenting with a history of epistaxis. Contrast enhanced computed tomography of paranasal sinuses revealed an intensely enhancing soft-tissue mass in the left nasal cavity and left middle and inferior meati with no obvious bony remodeling or destruction. We present imaging and pathologic features of nasal lobular capillary hemangioma and differentiate it from other entities like nasal angiofibroma.

Correlation of ultrasonographic parameters with serum creatinine in chronic kidney disease.

OBJECTIVE: The purpose of our study is to correlate renal echogenicity with serum creatinine in order to determine the significance of renal echogenicity when it comes to identifying the progression of chronic kidney disease (CKD) and for the sonographic grading of CKD. MATERIALS AND METHODS: Sixty patients above 30 years of age who had been diagnosed with CKD according to the guidelines of the National Kidney Foundation were included in the study. Patients on kidney replacement therapy or with fatty liver findings on ultrasonography were excluded. Ultrasounds of kidneys were performed by two radiologists who were blind to the patients' serum creatinine levels. Renal cortical echogenicity was compared with serum creatinine. Statistical analysis was performed using one-way ANOVA followed by Scheffe's test. The relationship between serum creatinine and sonographic features was assessed by correlation coefficient analysis. A P value less than 0.05 was considered statistically significant. RESULTS: Mean serum creatinine was 2.80 mg/dl for Grade 1 (range: 0.9-9.2 mg/dl), 3.69 mg/dl for Grade 2 (range: 1.2-10.3 mg/dl), 3.86 mg/dl for Grade 3 (range: 1.1-6.5 mg/dl), and 7.90 mg/dl for Grade 4 (range: 3.1-11.4 mg/dl). The grades being determined by cortical echogenicity on imaging A statistically significant, positive correlation was observed between serum creatinine and grading based on cortical echogenicity (P = 0.004). CONCLUSION: Renal echogenicity and its grading correlates better with serum creatinine in CKD than other sonographic parameters such as longitudinal size, parenchymal thickness, and cortical thickness. Hence, renal echogenicity is a better parameter than serum creatinine for estimating renal function in CKD, and has the added advantage of irreversibility.

Appendiceal Carcinoma with Krukenberg's Tumour Mimicking Primary Ovarian Cancer.

Appendiceal adenocarcinoma (AACa) is a rare tumour which represents 0.5% of all gastrointestinal malignancies. The prognosis is poor, because it is usually found at an advanced stage, that in turn, is partly due to a low threshold of suspicion and difficulties in diagnosis prior to surgery. It may occasionally demonstrate ovarian metastases that are large and which dominate the clinical and radiological presentations, leading to a misdiagnosis of an ovarian primary malignancy. We are reporting a case of an occult AACa which manifested clinically as a primary ovarian cancer which was at an advanced stage. Staging laparatomy revealed large bilateral ovarian tumours of clinical FIGO Stage III, with presumed appendiceal implants. Histological examination revealed a mucinous adenocarcinoma with a signet ring component, which involved bilateral ovaries and the appendix transmurally. Immunophenotypic analysis revealed a positive expression of CK 20 and CDX 2 and absence of CK 7 staining, which was compatible with appendiceal primary and ovarian metastases. The diagnosis was subsequently revised to AACa with Krukenberg's metastasis, Stage IV. Although AACas are uncommon, they should be considered in the differential diagnosis of intraabdominal masses and the distinction between ovarian and appendiceal primary malignancies is critical, as the treatment modalities vary.

Imaging in classic form of maple syrup urine disease: a rare metabolic central nervous system.

Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder of branched-chain amino acid metabolism. The condition gets its name from the distinctive sweet odour of affected infants' urine. MSUD is caused by a deficiency of the branched-chain α-ketoacid dehydrogenase enzyme complex, leading to accumulation of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. Imaging is characterestized by MSUD oedema affecting the myelinated white matter. We present a neonate with classic type of MSUD and its imaging features on computed tomography, conventional magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance spectroscopy.

Congenital cystic lung diseases.

Congenital cystic diseases of the lung are a rare but significant cause of morbidity in children and young adults presenting with respiratory distress and repeated chest infections. They consist of cystic adenomatoid malformation, bronchogenic cyst, pulmonary sequestration, and congenital lobar emphysema. Surgical treatment is a safe and an effective method of treatment. Chest X-ray and computed tomography are the key imaging modalities used for diagnosis.

Mixed adenoneuroendocrine carcinoma of cecum: a rare entity.

Mixed adenoneuroendocrine carcinoma of cecum (MANEC) was first reported by Cardier in 1924. These tumors are thought to arise from multi-potential stem cells, which have differentiated bidirectionally. Location of the tumor influences the treatment and outcome. We report a rare case of MANEC where the patient presented with abdominal pain and distension. Imaging revealed an ileo colic intussusception with the lead point being a MANEC.

A Rare Case of Primary osteosarcoma of urinary bladder.

Extraskeletal osteosarcoma is a malignant mesenchymal soft tissue tumor without attachment to the bone, and is able to produce osteoid or cartilaginous matrix. This entity accounts for 1% of all soft tissue sarcomas. Thus far, less than 35 cases of bladder osteosarcomas have been reported in the literature. These tumors are associated with very poor prognosis. We report a case of primary osteosarcoma of the urinary bladder presenting with intermittent hematuria, dysuria, and right flank pain. Contrast-enhanced computed tomography scan of the abdomen, pelvis, and chest revealed a bladder mass and pulmonary metastases with specks of calcification.